Valvular Abnormalities Research Articles

Bicuspid aortic valve: Basics and beyond.

Bicuspid aortic valve, one of the most common congenital cardiac malformations, is associated with other valvular abnormalities and aortopathies. It increases the risk of thoracic aortic aneurysm and dissection, infective endocarditis, and sudden cardiac death. Patients require regular surveillance for progressive valvular disease and aortopathy. Although bicuspid aortic valve may occur sporadically, many cases are inherited, so first-degree relatives should be evaluated and genetic counseling considered.

Value the Journey.

Value the Journey.

Exercise recommendations in patients with valvular heart disease.

Valvular heart disease affects 1%-2% of young individuals, many of whom aspire to partake in competitive sport or high intensity recreational exercise. There are limited reports on the impact of intensive physical activity on the progression of valvular heart disease; therefore, current recommendations are based on consensus opinion. The management of exercising individuals with valvular heart disease requires a structured approach that incorporates several key factors including symptomatic status, functional capacity, type and nature of the valvular lesion, impact on ventricular structure and function and effect on pulmonary artery pressure. Asymptomatic individuals with minor valvular abnormalities may engage in all forms of competitive sport, whereas those with lesions of moderate severity may exercise intensively if an exercise stress test tailored to the relevant physical activity reveals good functional capacity without myocardial ischaemia, haemodynamic disturbances or arrhythmia. Symptomatic athletes and those with severe valvular heart disease, impaired ventricular function, pulmonary hypertension and arrhythmias should refrain from most competitive sports. Athletes with a bicuspid aortic valve and aortic root diameter >40 mm should avoid sport with a strong isometric component even with minimal valvular dysfunction. There is an association between mitral valve prolapse and sudden cardiac death in the general population; however, there is limited evidence of increased risk with competitive sport. Athletes undergoing corrective surgery may return to exercise after 3 months if ventricular function and exercise capacity are preserved. Individuals anticoagulated for mechanical bioprosthetic valves should avoid contact or collision sport to minimise the risk of bleeding.

Streptobacillus moniliformis mitral valve endocarditis and septic arthritis: the challenges of diagnosing rat-bite fever endocarditis.

Streptobacillus moniliformis, the cause of rat-bite fever (RBF) in the United States, has rarely been reported as a cause of infectious endocarditis. In the majority of previously reported cases, the diagnosis was clinically- based in patients with underlying valvular abnormalities in the setting of positive blood culture for Streptobacillus moniliformis. We report a case of native valve endocarditis secondary to Streptobacillus moniliformis in a woman with a mitral valve vegetation but negative blood cultures where the diagnosis was established using molecular diagnostics on the valvular tissue.

Outcomes of Cardiac Screening in Adolescent Soccer Players

BackgroundReports on the incidence and causes of sudden cardiac death among young athletes have relied largely on estimated rates of participation and varied methods of reporting. We sought to investigate the incidence and causes of sudden cardiac death among adolescent soccer players in the United Kingdom.MethodsFrom 1996 through 2016, we screened 11,168 adolescent athletes with a mean (±SD) age of 16.4±1.2 years (95% of whom were male) in the English Football Association (FA) cardiac screening program, which consisted of a health questionnaire, physical examination, electrocardiography, and echocardiography. The FA registry was interrogated to identify sudden cardiac deaths, which were confirmed with autopsy reports.ResultsDuring screening, 42 athletes (0.38%) were found to have cardiac disorders that are associated with sudden cardiac death. A further 225 athletes (2%) with congenital or valvular abnormalities were identified. After screening, there were 23 deaths from any cause, of which 8 (35%) were sudden deaths attributed to cardiac disease. Cardiomyopathy accounted for 7 of 8 sudden cardiac deaths (88%). Six athletes (75%) with sudden cardiac death had had normal cardiac screening results. The mean time between screening and sudden cardiac death was 6.8 years. On the basis of a total of 118,351 person-years, the incidence of sudden cardiac death among previously screened adolescent soccer players was 1 per 14,794 person-years (6.8 per 100,000 athletes).ConclusionsDiseases that are associated with sudden cardiac death were identified in 0.38% of adolescent soccer players in a cohort that underwent cardiovascular screening. The incidence of sudden cardiac death was 1 per 14,794 person-years, or 6.8 per 100,000 athletes; most of these deaths were due to cardiomyopathies that had not been detected on screening. (Funded by the English Football Association and others.)

Anesthesiology

This concise book is intended to be a comprehensive, easy-to-read text aimed both at anesthesiology trainees preparing for examinations and experienced practitioners needing a succinct reference guide. Authored by a number of experienced practitioners in the field of anesthesiology from across the United States, the editors have structured this textbook to describe common challenges in clinical anesthesia in a question–answer format. The book comprises 12 main, specialty-specific sections and a total of 60 chapters. Each chapter starts with a hypothetical clinical scenario relevant to a particular subspecialty in anesthesiology. A large number of chapters address the cardiovascular system and anesthetic considerations, for example, ischemic heart disease, valvular abnormalities, or uncommon conditions such as care of a patient with a transplanted heart presenting for noncardiac surgery. In every chapter, the starting clinical scenarios are well structured and provide a comprehensive account of the clinical issue, including relevant investigations. The main content of each chapter is in a format of a series of simple questions and succinct answers. Wherever indicated, basic understanding of anatomy and physiology is discussed, such as applied anatomy of coronary circulation, the denervated heart or nomenclature, and classification of pacemakers. The anesthetic implications in each of these situations are covered explicitly and referenced appropriately. The style of presentation is one of the main strengths of this book. Using a problem-based approach, the authors immediately direct readers to the matter at hand, that is, the clinical situation and how to safely provide anesthesia care while facilitating critical thinking and problem-solving skills. The questions guide readers through the logical steps in safe anesthetic management of various elective and emergency cases in great detail. Current evidence and guidelines are cited wherever necessary, such as the 2014 ACC/AHA Guidelines on Perioperative Cardiovascular Evaluation and Management of Patients Undergoing Noncardiac Surgery,1 or the Universal Definition of Myocardial infarction.2 Chapters on intraoperative coagulopathies, difficult airway management, and obstetric anesthesia also cover the applied pathophysiology and anesthetic implications quite well. The section on pediatric anesthesiology topics is comprehensive, and particularly, neonatal resuscitation and premature infant are described. Because this is the first edition of this book, it can be appraised using Yao and Artusio’s Anesthesiology: Problem-Oriented Patient Management,3 a core textbook, now in its 8th edition. Both books are laid out in a problem-based format using clinically relevant case scenarios. The table of contents is similar; both books deal with the topics in a well-organized questionanswer format. The Yao and Artusio book is a major reference text and covers each topic in extensive detail, with preoperative, intraoperative, and postoperative phases clearly yet compactly presented, referenced, and well illustrated where appropriate. The Anesthesiology case reviews book touches on the most relevant topics; however, the level of detail is not as extensive as that of the Yao and Artusio book. Some important and current practices in anesthesiology have been overlooked in this book, such as important considerations for use of newer reversal agents for nondepolarizing neuromuscular blockers (sugammadex) or use of gastric ultrasound for patients with a full stomach. Moreover, obstructive sleep apnea is not described as a condition in its own right, but as an association with obesity. The recent updated STOP-Bang questionnaire4 and the 2016 Society of Anesthesia and Sleep Medicine Guidelines on Preoperative Screening and Assessment of Adult Patients with Obstructive Sleep Apnea5 are also missing. In the regional anesthesia chapters, brachial plexus anatomy illustrations are presented in color; however, there is limited mention of the use of ultrasound for nerve blocks, a standard of care practiced in many institutions worldwide. The addition of colorful illustrations, graphs, and figures could make this book more readable. Some integral anesthesia topics such as acute and chronic pain management are mentioned very superficially, and some readers may find this an important exclusion of a significant knowledge area. Overall, this is a good book, serving as a handy resource for both trainees and clinicians who like to review high-yield topics in a case-based format. This book finds its place somewhere between being a useful concise pocket handbook for bedside reference, such as Oxford Handbook for Anesthesia,6 and a case-based reference book with more detail, such as the Yao and Artusio book.3 In the end, if a reader would like to use a textbook that touches on most of the relevant clinical topics in anesthesia or prepare for examinations, this book will serve as an important addition to their collection of useful resources. Najia Hasan, MBBS, FRCADepartment of Anaesthesia and Intensive CarePrince Charles HospitalCwm Taf University Health BoardWales, United Kingdom Mandeep Singh, MBBS, MD, MSc, FRCPCDepartment of Anesthesiology and Pain ManagementToronto Western HospitalUniversity Health NetworkUniversity of TorontoToronto, Ontario, CanadaDepartment of Anaesthesiology and Pain ManagementWomen’s College HospitalToronto, Ontario, CanadaToronto Sleep and Pulmonary CentreToronto, Ontario, Canada[email protected]

OBESITY AS ENVIRONMENTAL RISK FACTOR FOR REPEATED PREGNANCY LOSS IN PATIENTS WITH ANTIPHOSPHOLIPID SYNDROME

Introduction: Antiphospholidsyndrome (APS) is an autoimmune hypercoagulablestate caused by antiphospholid antibodies. Primary antiphospholipidsyndrome occurs in absence of any other related disease. Secondary antiphospholipid syndrome occurs with other autoimmune disease as systemic lupus erythrematosis. The clinical features of the APS are various, but the most common clinical presentation is pregnancy loss, DVT. Pulmonary thromboembolism, the cardiac manifestations of APS include myocardial infarction, pericardial effusion, myocardiopathy, and coronary artery thrombosis, but the most common manifestation is valvular abnormalities. Aim: to investigate the influence of obesity in women diagnosed as an antiphospholipid cases according to Sydney’s criteria on pregnancy morbidities, vascular events, pulmonary thromboemboism and laboratory criteria. Methods: A case control study was conducted that include women attending obstetric outpatient clinic. These women were previously diagnosed as antiphospholipid cases according to (Sydney’s criteria).A total of 60 women have been equally divided into two groups: First group corresponds to control group which includes 30 women with normal body mass index (BMI) i.e. 30Kg/m2). Results: Obesity is independent predictor for preterm birth intrauterine fetal death and pulmonary thromboembolism. Conclusion: Obesity is associated with worth pregnancy outcomes in patients with primary antiphospholipid syndrome.

Severe Maternal Morbidity and Mortality in Women with Congenital Heart Disease [18R

INTRODUCTION: Studies have demonstrated an increase in adverse pregnancy outcomes in women with congenital heart disease. We sought to examine the association of maternal congenital heart disease (CHD) with the risk of severe maternal morbidity and mortality (SMM) compared to women without congenital heart disease. METHODS: This retrospective cross-sectional study used 2008 through 2012 New York City (NYC) birth certificates linked to hospital discharge data to identify delivery hospitalizations. Cases of SMM were identified using ICD-9 diagnosis and procedure codes based on the Center for Disease Control and Prevention criteria. Data were limited to singleton, live births. CHD included women with any vascular, septal, or valvular abnormality of the heart at birth. Multivariable logistic regression was used to evaluate SMM in women with CHD adjusting for maternal demographics, socioeconomic status, and medical comorbidities. RESULTS: From 2008 through 2012 there were 578,840 live singleton births in NYC, 347 of these births were to women with CHD (6/10,000). Acute renal failure, adult respiratory distress syndrome, thrombotic embolism, cardiac arrest/ventricular fibrillation, pulmonary edema/heart failure, conversion of cardiac rhythm, and transfusion were all independently associated with CHD. Women who had CHD had higher adjusted odds of SMM(aOR 2.43; 95% CI 1.60-3.70) compared to women without CHD after adjusting for maternal socioeconomic status, demographics and medical comorbidities. There was one maternal death. CONCLUSION: SMM is increased in women with CHD, even after adjusting for co-morbid conditions. It is important that these women receive care from an identified multidisciplinary team from preconception to postpartum to optimize pregnancy outcomes.

Natural history of echocardiographic abnormalities in mucopolysaccharidosis III

BackgroundMucopolysaccharidosis (MPS) type III, Sanfilippo Syndrome, is an autosomal recessive lysosomal storage disorder. MPS I and II patients often develop cardiac involvement leading to early mortality, however there are limited data in MPS III. The objective of this study is to describe cardiac abnormalities in a large group of MPS III patients followed in a longitudinal natural history study designed to determine outcome measures for gene transfer trials. MethodsA single center study of MPS III patients who were enrolled in the Nationwide Children's Hospital natural history study in 2014. Two cardiologists reviewed all patient echocardiograms for anatomic, valvular, and functional abnormalities. Valve abnormalities were defined as abnormal morphology, trivial mitral regurgitation (MR) with abnormal morphology or at least mild MR, and any aortic regurgitation (AR). Abnormal left ventricular (LV) function was defined as ejection fraction < 50%. Group comparisons were assessed using two-sample t-tests or Wilcoxon rank sum tests for continuous variables and chi-square or Fisher's exact tests for categorical variables. ResultsTwenty-five patients, 15 Type A and 10 Type B MPS III, underwent 45 echocardiograms. Fifteen patients (60%) demonstrated an abnormal echocardiographic finding with age at first abnormal echocardiogram within the study being 6.8 ± 2.8 years. Left-sided valve abnormalities were common over time: 7 mitral valve thickening, 2 mitral valve prolapse, 16 MR (8 mild, 8 trivial), 3 aortic valve thickening, and 9 AR (7 mild, 2 trivial). Two patients had asymmetric LV septal hypertrophy. No valvular stenosis or ventricular function abnormalities were noted. Incidental findings included: mild aortic root dilation (2), bicommissural aortic valve (1), and mild tricuspid regurgitation (3). ConclusionsIndividuals with Sanfilippo A and B demonstrate a natural history of cardiac involvement with valvular abnormalities most common. In short-term follow up, patients demonstrated only mild progression of abnormalities, none requiring intervention. Valvular disease prevalence is similar to MPS I and II, but appears less severe. These findings raise no specific concerns for gene transfer trials in patients in this age range.

An Unusual Case of Tricuspid Stenosis.

Tricuspid stenosis is an uncommon valvular abnormality commonly associated with other valvular lesions. Ebstein’s anomaly is a rare congenital heart malformation characterized primarily by abnormalities of the tricuspid valve and right ventricle. Endomyocardial fibrosis is a restrictive cardiomyopathy observed in tropical and subtropical regions. It may cause right ventricular distortion with apparent apical displacement of the tricuspid valve, mimicking Ebstein’s anomaly. Eosinophilia is the most commonly cited aetiological link in endomyocardial fibrosis. Here we report the case of 42-year-old male patient who presented with heart failure and severe tricuspid stenosis where a diagnosis of hypereosinophilic syndrome was also established. This case represented a diagnostic challenge in the search for the definitive cause of the tricuspid stenosis.LEARNING POINTSEbstein’s anomaly is a rare congenital heart malformation characterized primarily by abnormalities of the tricuspid valve and right ventricle. The tricuspid valve is usually incompetent, and very rarely stenotic.Hypereosinophilic syndromes can be associated with heart damage. The fibrotic stage of eosinophil-mediated heart damage is characterized by altered cardiac function due to either compromise/entrapment of the cordae tendineae and/or restrictive cardiomyopathy.Endomyocardial fibrosis is a restrictive cardiomyopathy observed in tropical and subtropical regions that may be indistinguishable from the Loeffler’s endocarditis observed in temperate climates. It may cause right ventricle distortion and apical displacement of the tricuspid valve, mimicking Ebstein’s anomaly.

Menarche, menopause, and pregnancy data in untreated females and females treated with agalsidase alfa in the Fabry Outcome Survey

Mucopolysaccharidosis (MPS) type III, Sanfilippo Syndrome, is an autosomal recessive lysosomal storage disorder. MPS I and II patients often develop cardiac involvement leading to early mortality, however there are limited data in MPS III. The objective of this study is to describe cardiac abnormalities in a large group of MPS III patients followed in a longitudinal natural history study designed to determine outcome measures for gene transfer trials.A single center study of MPS III patients who were enrolled in the Nationwide Children's Hospital natural history study in 2014. Two cardiologists reviewed all patient echocardiograms for anatomic, valvular, and functional abnormalities. Valve abnormalities were defined as abnormal morphology, trivial mitral regurgitation (MR) with abnormal morphology or at least mild MR, and any aortic regurgitation (AR). Abnormal left ventricular (LV) function was defined as ejection fraction < 50%. Group comparisons were assessed using two-sample t-tests or Wilcoxon rank sum tests for continuous variables and chi-square or Fisher's exact tests for categorical variables.Twenty-five patients, 15 Type A and 10 Type B MPS III, underwent 45 echocardiograms. Fifteen patients (60%) demonstrated an abnormal echocardiographic finding with age at first abnormal echocardiogram within the study being 6.8 ± 2.8 years. Left-sided valve abnormalities were common over time: 7 mitral valve thickening, 2 mitral valve prolapse, 16 MR (8 mild, 8 trivial), 3 aortic valve thickening, and 9 AR (7 mild, 2 trivial). Two patients had asymmetric LV septal hypertrophy. No valvular stenosis or ventricular function abnormalities were noted. Incidental findings included: mild aortic root dilation (2), bicommissural aortic valve (1), and mild tricuspid regurgitation (3).Individuals with Sanfilippo A and B demonstrate a natural history of cardiac involvement with valvular abnormalities most common. In short-term follow up, patients demonstrated only mild progression of abnormalities, none requiring intervention. Valvular disease prevalence is similar to MPS I and II, but appears less severe. These findings raise no specific concerns for gene transfer trials in patients in this age range.

Abstract 123: Diagnostic Yield of TEE in Patients With Cryptogenic Stroke and TIA With Normal TTE: A Systematic Review and Meta-Analysis

Background: The diagnostic utility of Transesophageal echocardiography (TEE) in cryptogenic stroke or TIA remains controversial. Objective: To perform a systematic review and meta-analysis to estimate an additional diagnostic yield and clinical impact of TEE in patients with cryptogenic stroke and TIA with normal Transthoracic Echocardiography (TTE) results. Methods: A PubMed literature review was performed of cohort studies. The studies were excluded if routine stroke work-up was not documented, TTE was not performed on all patients or clear TTE data not available, separate analysis for patients with normal TTE results were not available, did not report all cardiac TEE findings, and included patients with Afib or did not have a separate analysis for patients without Afib. Results: Fifteen studies met the criteria, with total of 2054 patients with cryptogenic stroke or TIA with normal TTE results and underwent TEE. All studies were evaluated for internal validity and indirectness. Following TEE findings were noted across different studies: LA/LAA/Aortic thrombus, valvular vegetation, intracardiac tumor, spontaneous echo contrast, PFO, ASA, PFO -ASA combination, valvular abnormalities and complex aortic plaques. 37.5% (29.7% - 45.1%) of patients had additional cardiac findings on TEE. PFO was the second most common abnormality noted after complex aortic plaque. 13.6% (8.1% - 19.1%) of patients required changes in the management after TEE evaluation. Based on the current guidelines, it should change management in 4.1% (2.1% - 6.2%) of patients. It could potentially change management in 30.4% (21.9% - 38.9%) of patients. Analysis was also performed with only class II studies to increase internal validity, which showed additional cardiac findings in 38.4% (28.5% - 48.3%), changed management in 20.2 % (8.7% - 31.8%), should change management in 4.7% (1.5% - 7.9%) and could potentially change management in 30.4% (17.8% - 43.0%) of patients. Conclusions: The diagnostic yield of TEE to find any additional cardiac findings in patients with cryptogenic stroke or TIA is approximately 38%. Based on this analysis, number needed to diagnose one additional abnormality with TEE that can change management per current guidelines is approximately 21.

Examination of Pituitary Adenylate Cyclase-Activating Polypeptide (PACAP) as a Potential Biomarker in Heart Failure Patients.

Pituitary adenylate cyclase-activating polypeptide (PACAP) is a pleiotropic and multifunctional neuropeptide having neurotrophic, neuroprotective, and general cytoprotective actions in a variety of tissues based on its anti-apoptotic, anti-inflammatory, and antioxidant effects. Several studies have demonstrated its cardioprotective effects in vitro and in various animal models. However, few data are available on the presence of PACAP in human cardiac tissues and its role in the pathomechanism and progression of different cardiac disorders, particularly heart failure. Earlier, our research group has shown PAC1 receptor immunoreactivity in human heart tissue samples and we have found significantly elevated PACAP27- and PACAP38-like immunoreactivity in ischemic cardiac samples compared to valvular abnormalities with radioimmunoassay. In the last few years, numerous studies examined the presence and the changes of PACAP levels in different human tissue samples and biological fluids to show alterations in different physiological and pathological conditions. Therefore, the aim of the present study was to measure the alterations of blood PACAP levels in chronic heart failure caused by primary dilated cardiomyopathy or ischemic cardiomyopathy and to examine the possible relationship between serum levels of PACAP, N-terminal prohormone of brain natriuretic peptide (NT-proBNP), and systolic left ventricular function, the most reliable biomarkers of heart failure. In the group of mild heart failure patients, a significant strong negative correlation was detected. Furthermore, in moderate heart failure, we found a significant moderate negative correlation between PACAP and NT-proBNP levels only in ischemic subgroup. Positive correlation was found between serum PACAP level and ejection fraction only in patients with heart failure due to ischemic cardiomyopathy but not in patients with primary dilated cardiomyopathy. In summary, remarkable differences were observed between the ischemic and non-ischemic heart failure suggesting that PACAP might play an important role in the pathomechanism and progression of ischemic heart failure and it might be a potential biomarker of cardiac diseases in the future.

EGFR activation triggers cellular hypertrophy and lysosomal disease in NAGLU-depleted cardiomyoblasts, mimicking the hallmarks of mucopolysaccharidosis IIIB

Mucopolysaccharidosis (MPS) IIIB is an inherited lysosomal storage disease caused by the deficiency of the enzyme α-N-acetylglucosaminidase (NAGLU) required for heparan sulfate (HS) degradation. The defective lysosomal clearance of undigested HS results in dysfunction of multiple tissues and organs. We recently demonstrated that the murine model of MPS IIIB develops cardiac disease, valvular abnormalities, and ultimately heart failure. To address the molecular mechanisms governing cardiac dysfunctions in MPS IIIB, we generated a model of the disease by silencing NAGLU gene expression in H9C2 rat cardiomyoblasts. NAGLU-depleted H9C2 exhibited accumulation of abnormal lysosomes and a hypertrophic phenotype. Furthermore, we found the specific activation of the epidermal growth factor receptor (EGFR), and increased phosphorylation levels of extracellular signal-regulated kinases (ERKs) in NAGLU-depleted H9C2. The inhibition of either EGFR or ERKs, using the selective inhibitors AG1478 and PD98059, resulted in the reduction of both lysosomal aberration and hypertrophy in NAGLU-depleted H9C2. We also found increased phosphorylation of c-Src and a reduction of the hypertrophic response in NAGLU-depleted H9C2 transfected with a dominant-negative c-Src. However, c-Src phosphorylation remained unaffected by AG1478 treatment, posing c-Src upstream EGFR activation. Finally, heparin-binding EGF-like growth factor (HB-EGF) protein was found overexpressed in our MPS IIIB cellular model, and its silencing reduced the hypertrophic response. These results indicate that both c-Src and HB-EGF contribute to the hypertrophic phenotype of NAGLU-depleted cardiomyoblasts by synergistically activating EGFR and subsequent signaling, thus suggesting that EGFR pathway inhibition could represent an effective therapeutic approach for MPS IIIB cardiac disease.

Thoracic aortic aneurysm in patients with loss of function Filamin A mutations: Clinical characterization, genetics, and recommendations.

The frequency and gender distribution of thoracic aortic aneurysm as a cardiovascular manifestation of loss-of-function (LOF) X-linked FilaminA (FLNA) mutations are not known. Furthermore, there is very limited cardiovascular morbidity or mortality data in children and adults. We analyzed cardiac data on the largest series of 114 patients with LOF FLNA mutations, both children and adults, with periventricular nodular heterotopia (PVNH), including 48 study patients and 66 literature patients, median age of 22.0 years (88 F, 26 M, range: 0-71 years), with 75 FLNA mutations observed in 80 families. Most (64.9%) subjects had a cardiac anomaly or vascular abnormality (80.8% of males and 60.2% of females). Thoracic aortic aneurysms or dilatation (TAA) were found in 18.4% (n = 21), and were associated with other structural cardiac malformations in 57.1% of patients, most commonly patent ductus arteriosus (PDA) and valvular abnormalities. TAA most frequently involved the aortic root and ascending aorta, and sinus of Valsalva aneurysms were present in one third of TAA patients. Six TAA patients (28.5%) required surgery (median age 37 yrs, range 13-41 yrs). TAA with its associated complications was also the only recorded cause of premature, non-accidental mortality in adults (2 M, 2 F). Two adult patients (1 F, 1 M, median 38.5 yrs), died of spontaneous aortic rupture at aortic dimensions smaller than current recommendations for surgery for other aortopathies. Data from this largest series of LOF FLNA mutation patients underscore the importance of serial follow-up to identify and manage these potentially devastating cardiovascular complications.

Competence of Intensivists in Focused Transthoracic Echocardiography in Intensive Care Unit: A Prospective Observational Study.

Objectives:Focused transthoracic echocardiography (fTTE) in critical care can be used to assess patient's volume status, ventricular contractility, right ventricle chamber size, and valvular abnormalities. The objective of the study was to assess the competency of intensivists in performing fTTE in Intensive Care Unit (ICU) patients after a brief training course by cardiologist using a specific ECHO protocol.Methods:One hundred and four patients in ICU were recruited for this prospective observational study over a period of 12 months. Intensivists were trained for 60 h (2 h/day for 30 days). Intensivists performed fTTE in 82 ICU patients using a specific ECHO protocol developed in consensus with cardiologists. Each patient was assessed by an intensivist and two blinded cardiologists. At the end of the study period, the competency of intensivists was compared with two cardiologists and analyzed using intraclass correlation coefficient (ICC).Results:There were excellent agreement between intensivists and cardiologists in terms of measuring ejection fraction (ICC estimate was 0.973–0.987), valvular function (ICC estimate for mitral valve was 0.940–0.972; ICC estimate for aortic valve was 0.872–0.940), and ICC estimate for pulmonary hypertension was 0.929–0.967. Good reliability has been found for the assessment of volume status with inferior vena cava diameter (ICC estimate for assessing hypovolemia was 0.790–0.902).Conclusion:Intensivists with requisite training in TTE were able to perform focused echocardiography with comparable accuracy to that of cardiologists. Further studies are required to elucidate the therapeutic implications of fTTE performed by the intensivists.

Functional Tricuspid Regurgitation and Ring Annuloplasty Repair

Functional tricuspid regurgitation (TR) primarily arises from asymmetric dilation of the tricuspid annulus in the setting of right ventricular dysfunction and enlargement in response to left-sided myocardial and valvular abnormalities. Even if the TR is not severe at the time of mitral valve surgery, it can worsen and even appear late after successful mitral valve surgery, which portends a poor prognosis. Despite data demonstrating inferior outcomes in the presence of residual TR, surgical repair for functional TR remains underused. Acceptance of TR, in the presence of tricuspid annular dilation, may be unacceptable. Surgical repair should consist of placement of a rigid or semirigid annular ring, which has been shown to provide superior durability as compared with suture and flexible band techniques. Finally, percutaneous annuloplasty for correction of functional TR may allow treatment of patients with recurrent TR at high risk of reoperation.

Management of Mitral Regurgitation in a Patient Contemplating Pregnancy

Management of rheumatic mitral regurgitation in a woman contemplating pregnancy presents unique challenges for the clinician. When tasked with taking care of this type of patient, attention needs to be paid to the patient’s functional status to determine if symptoms are present. In addition to this clinical assessment, transthoracic echocardiography is also critical. It provides insight into the etiology of the mitral regurgitation, assesses for the presence of concomitant mitral stenosis or other valvular abnormalities, characterizes the severity of mitral regurgitation through an integrative approach and identifies high risk findings including progressive left ventricular (LV) dilation and LV dysfunction. Surgical intervention is recommended for symptomatic patients and in asymptomatic patients with evidence of progressive LV dilation and a LV ejection fraction of less than 60%. While the presence of pulmonary hypertension and atrial fibrillation have been shown to be risk factors in degenerative mitral regurgitation, the same has not been demonstrated in rheumatic mitral valve disease. While mitral regurgitation may be reasonably well tolerated during pregnancy, symptomatic patients are at higher risk for adverse maternal and fetal outcomes, and therefore, it is recommended that mitral valve surgery be performed prior to pregnancy. Once the decision has been made to proceed to surgery, mitral repair, performed at a Heart Valve Center of Excellence is recommended if possible due to improved outcomes. Mitral valve repair is possible in &gt;80% cases of rheumatic mitral regurgitation. If repair is not possible, replacement with either a bioprosthetic or mechanical valve are reasonable options. There are advantages and disadvantages to each approach and the choice of prosthesis should be a shared decision between the patient and her treatment team.

Staphylococcus aureus Myocarditis: Case Report and Literature Review

Staphylococcus aureus is widely reported to be the most common bacterial cause of myocarditis, and usually occurs in the setting of bacteraemia and sepsis. Rarely, it occurs without associated infective endocarditis. Previous case reports of S. aureus myocarditis occur almost exclusively in immunocompromised patients. We report a case of S. aureus myocarditis with associated inflammatory thrombus in an immunocompromised patient. A 45-year-old man with a previous history of rheumatoid arthritis on long-term methotrexate presented with septic shock. He was found to have a methicillin-sensitive S. aureus bacteraemia. The patient subsequently developed olecranon bursitis requiring surgical wash out. Transthoracic echocardiogram revealed a very atypical echodense mass in the left ventricular apex, with normal systolic function and no associated valvular abnormalities. Cardiac magnetic resonance imaging showed oedema in the endomyocardium, consistent with acute myocarditis, associated with an apical mural thrombus. Repeat cardiac magnetic resonance 3 weeks following discharge from hospital showed marked improvement in endomyocardial oedema and complete resolution of the apical mural thrombus. He was treated with a 12-week course of antibiotics and anticoagulated with apixaban. Staphylococcal myocarditis without endocarditis is a rare disease entity, and significant disease manifestations can arise even without valvular involvement. Cardiac magnetic resonance is a useful diagnostic tool in bacterial myocarditis and characterisation of apical masses detected on transthoracic echocardiogram. The role of direct acting oral anticoagulants in this context remains unclear.

Should the dilated ascending aorta be repaired at the time of bicuspid aortic valve replacement?

Bicuspid aortic valve (BAV) is the most common congenital valvular abnormality and frequently presents with accelerated calcific aortic valve disease, requiring aortic valve replacement (AVR) and thoracic aortic aneurysm and dissection. Supporting evidence for Association Guidelines of aortic dimensions for aortic resection is sparse. We sought to determine whether concurrent repair of dilated or aneurysmal aortic disease during AVR in patients with BAV substantially improves morbidity and mortality outcomes. Mortality and reoperation outcomes of 1301 adults with BAV and dilated aorta undergoing AVR-only surgery were compared to patients undergoing AVR with aortic resection (AVR-AR) using Cox proportional hazards modelling and patient matching. Clinically important differences in patient characteristics, aortic valve function and aortic dimensions were identified between cohorts. Event rates were low, with rates of reoperation and death within 1 year of only 1.8% and 5.4%, respectively, and no aortic dissection observed during follow-up. There were no significant differences in reoperation or mortality outcomes between the AVR-only and AVR-AR cohorts. Age, aortic dimension or a combination thereof was not associated with better or worse outcomes after each AVR-AR compared with AVR. We conclude AVR-only and AVR-AR surgery have low morbidity and mortality and have utility over a wide range of age and aortic sizes. Our results do not provide support for the 45-mm aortic dimension recommended in the current guidelines for aortic resection while performing AVR or any other specific dimension.